Bochdalek diaphragmatic hernia usually begins during childhood, but may be an occasional finding even in adults. The treatment of choice is surgical repair to avoid herniated bowel complications. The operation often requires a combined approach consisting in thoracotomy and laparotomy. This is a convenient solution to eliminate the vascular risks (if there are additional concomitant embryonic defects, such as intestinal malrotation). We report a case of a female, aged 45 years, with epigastric cramp-like pain for 4 days and tenderness in the right abdominal quadrants during physical examination; the standard laboratory data showed decreased blood levels of calcium and potassium. Chest and abdominal X-rays revealed significant, widespread colic distension and the presence of a colic loop in the chest. We confirmed these results by CT and barium enema and proceeded with urgent surgery consisting in a right hemicolectomy (extended as far as the left part of the tranverse colon) for volvulus and with stitching of the diaphragmatic gap. We also discovered incomplete intestinal malrotation. After surgery, complete remission of the clinical symptoms was achieved. This case report demonstrates that, despite the apparent clinical silence, congenital diaphragmatic hernia in an adult may often manifest itself with particular gravity calling for urgent surgery.